12 research outputs found
Central Nervous System Involvement of Epstein Barr Virus Associated Lymphoproliferative Disorder in a Child with Acute Lymphoblastic Leukemia: Successful Treatment with Rituximab and Interferon-Alpha
Congenital Complete Tracheal Ring in a Neonate: A Case Report
Abnormalities of the upper airway tract lead to congenital high airway obstruction and may complicate neonatal airway management in the delivery room. Congenital complete tracheal rings are a rare and unusual tracheal anomaly, usually presenting in the neonate or infant as respiratory distress. The clinical presentation can vary from almost asymptomatic patients to near-fatal airway obstruction. It may exist as an isolated entity, or in association with other congenital malformations, in particular, cardiac anomalies along with vascular rings and pulmonary slings. Other associated anomalies have also been reported, for example, chromosomal anomalies, malformation of other parts of the respiratory tract, esophagus and skeletal systems. Here, we report an extreme case of VACTERL/TACRD association presented with congenital complete tracheal ring, encephalocele, bilateral radial agenesis with absent thumbs, equinovalgus deformity on right foot, low-set ears and micrognathia
Prune-Belly Sendromlu Bir Olguda Erken Prenatal Girişim ile Başarılı Tedavi
Prune-Belly Syndrome (PBS) is a congenital disorder characterized by abdominal wall musculature deficiency, urinary tract anomalies and bilateral cryptorchidism. Urinary tract disease is the most important prognostic factor with the complications of pulmonary hypoplasia and end stage renal disease resulting in a mortality rate of 60%. Although the mode of inheritance of PBS is unclear, it has been hypothesized that PBS has a genetic basis. in the antenatal period relieving urinary tract obstruction can improve the survival rate of patients with PBS. We report a neonate with familial prune-belly syndrome who was applied vesico-amniotic shunt and so was affected slightlyPrune-Belly Sendromu (PBS) abdominal kasların hipoplazisi, üriner system anomalileri ve bilateral inmemiş testis ile karakterize konjenital bir hastalıktır. Üriner sistem tutulumu %60 mortaliteye sahip olan son dönem böbrek hastalığı ve pulmoner hipoplazi komplikasyonları ile en önemli prognostik faktördür. PBS'nin kalıtım şekli bilinmemesine rağmen, hastalığın bir genetik temeli olduğu düşünülmektedir. Antenatal dönemde üriner sistemdeki obstrüksiyonun giderilmesi hastaların yaşam şansını arttırmaktadır. Biz prenatal dönemde vezikoamniyotik şant uygulanan ve böylece az etkilenen ailesel bir PBS olgusu sundu
Erken süt çocukluğu döneminde Crohn hastalığı: Olgu sunumu
Crohn disease is rare in infancy and early childhood. It is reported that patients with Crohn disease are usually diagnosed during school age and adolescence periods. Chronic diarrhea, failure to thrive, rectal bleeding, abdominal pain, fever, oral and perianal lesions and high levels of acute phase reactants suggest Crohn disease. Inflamatuar bowel diseases should be considered in cases with intractable amoebiasis. Inflammatory bowel diseases should be taken into account for differential diagnosis in cases with amoebiasis resistant to therapy.Crohn hastalığı süt çocukluğu döneminde sık görülmez. Çocukluk yaş grubunda olgular genelde okul çağı ve adolesan yaşlarda görülür. Kronik ishal, gelişme geriliği, rektal kanama, karın ağrısı, ateş, oral ve perianal lezyonların varlığı, yüksek akut faz reaktanlari Crohn hastalığını düşündüren bulgulardır. Öte yandan, amip tedavisine karşın iyileşmeyen amebiyazis olgularında, bu durumun inflamatuvar barsak hastalığına bağlı olabileceği akılda tutulmalı, tedaviye dirençli amip enfeksiyonlarında ayırıcı tanıda inflamatuvar barsak hastalıkları düşünülmeli ve araştırılmalıdır